Background

The association of postcricoid dysphagia, upper esophageal webs, and iron deficiency anemia is known as Plummer-Vinson syndrome (PVS) in the United States and Paterson-Brown Kelly syndrome in the United Kingdom.
[1, 2, 3, 4, 5] The term sideropenic dysphagia has also been used, because the syndrome can occur with iron deficiency (sideropenia), but it is not associated with anemia.
[6]

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Pathophysiology

The pathogenesis of Plummer-Vinson syndrome (PVS) remains speculative, and the existence of the syndrome has been challenged. Postulated etiopathogenic mechanisms include iron and nutritional deficiencies, genetic predisposition, and autoimmune factors, amongst others.
[7, 8]

The prevalent iron deficiency theory remains controversial. Older reports have implicated iron deficiency in the pathogenesis of esophageal webs and dysphagia in predisposed individuals. The depletion of iron-dependent oxidative enzymes may produce myasthenic changes in muscles involved in the swallowing mechanism, atrophy of the esophageal mucosa, and formation of webs as epithelial complications.

The improvement in dysphagia after iron therapy provides evidence for an association between iron deficiency and postcricoid dysphagia.
[2] Anecdotal reports have also been made of patients with PVS exhibiting impaired esophageal motility (with dysphagia) that recovers following iron therapy.
[9] Moreover, the decline in PVS seems to parallel an improvement in nutritional status, including iron supplementation.

However, population-based studies have shown no relationship between postcricoid dysphagia and anemia or sideropenia.
[6] Other studies have demonstrated that patients with webs are as likely to be iron deficient as controls, and webs are often found in patients without iron deficiency or dysphagia. Lastly, the iron deficiency theory does not explain the predilection of webs for the upper esophagus and the rarity of the syndrome in populations in which chronic iron deficiency is endemic (eg, eastern and central Africa).
[2]

PVS has also been viewed as an autoimmune phenomenon. The syndrome has been associated with autoimmune conditions, such as rheumatoid arthritis, pernicious anemia, celiac disease, and thyroiditis.
[10, 7] In one study, a significantly higher proportion of patients with PVS had thyroid cytoplasmic autoimmune antibodies compared to controls with iron deficiency. The autoimmune theory, however, has gained little acceptance to date.

A complicated inlet patch (heterotopic gastric mucosa) has also been implicated in the pathogenesis of PVS.
[11, 12] An ulcerated inlet patch in the upper esophagus can cause stricturing (weblike formation) and bleeding (with subsequent iron deficiency).
[13] However, most studies with biopsy or autopsy specimens have not demonstrated the presence of gastric metaplasia in the samples.

Epidemiology

United States data

Reliable prevalence data on PVS are lacking. The syndrome is now a rarity, and its decline has been attributed to better nutrition and health care. Webs may be found in 5-15% of patients presenting with dysphagia, but most of these patients do not have PVS.

International data

In the first half of the 20th century, PVS was a relatively common finding, particularly in middle-aged Scandinavian women. The rapid fall in prevalence of the syndrome in the latter part of the 20th century has paralleled an improvement in nutritional status, including widespread addition of iron to flour.
[15]

Race-, sex-, and age-related demographics

PVS has mainly been described in whites, and it is more frequently observed in women. In earlier studies from Scandinavia, up to 90% of patients were women.

The typical age range at diagnosis is 40-70 years. A handful of cases have been reported in children.
[16]

Mortality/Morbidity

Morbidity issues primarily relate to diet modification and repeat esophageal dilations (with a small risk of perforation) in patients with PVS who have recurrent dysphagia. Updated mortality data are unavailable for this rare syndrome, but, presumably, mortality is low.

Complications

Although reports are inconsistent, patients with PVS seem to be at an increased risk for hypopharyngeal and esophageal cancers. A high prevalence of hypopharyngeal cancers in Swedish women in the 1930s and 1940s was attributed to PVS.
[19]

The reported frequency of postcricoid carcinoma associated with PVS varies (4-16% in older studies) and remains a matter of debate.
[20]

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Patient Education

Instruct patients with PVS on dietary modifications and eating habits.

Educate patients with PVS about the potential association of PVS and hypopharyngeal and esophageal cancers to ensure appropriate follow-up care.